Skin nonmelanocytic tumor

Lymphoma and related disorders (see also Lymphoma chapter)

Cutaneous T cell lymphoma (CTCL)

Primary cutaneous anaplastic large cell lymphoma

Last author update: 27 February 2020
Last staff update: 17 May 2021

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PubMed search: Primary cutaneous anaplastic large cell lymphoma [title] pathology

Maria C. Ferrufino-Schmidt, M.D.
Roberto N. Miranda, M.D.
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Cite this page: Ferrufino-Schmidt MC, Marques-Piubelli ML, Miranda RN. Primary cutaneous anaplastic large cell lymphoma. website. Accessed September 26th, 2023.
Definition / general
Essential features
  • Primary cutaneous anaplastic large cell lymphoma is included under the umbrella term CD30+ T cell lymphoproliferative disorders (Am J Dermatopathol 2017;39:877)
ICD coding
  • ICD-O: 9718/3 - primary cutaneous anaplastic large cell lymphoma
Clinical features
Prognostic factors
Case reports
Clinical images

Contributed by Roberto N. Miranda, M.D.

Clinical presentation of C-ALCL

Images hosted on other servers:

Annular, erythematous
to violaceous indurated
plaques with fixed
nodules over arm
Gross description
Microscopic (histologic) description
  • Diffuse dermal infiltrates with cohesive sheets of tumor cells with anaplastic morphology (round to irregularly shaped nuclei, prominent eosinophilic nucleoli and abundant cytoplasm); nonanaplastic appearance (pleomorphic or immunoblastic) is seen in 20% of cases (Pathology 2020;52:100, Am J Dermatopathol 2017;39:877)
  • Epidermotropism is marked in cases with DUSP22-IRF4 rearrangement (Histopathology 2015;66:846)
  • Cerebriform lymphocytes are not identified
  • Variable degrees of inflammatory infiltrate, reactive lymphocytes are common in the periphery of the lesions (Br J Dermatol 2003;148:580)
  • Ulcerated lesions may have lymphomatoid papulosis-like histology with abundant reactive T cells, histocytes, eosinophils, neutrophils and a low number of CD30+ cells (Am J Surg Pathol 2014;38:1203; J Cutan Pathol 2015;42:610)
Microscopic (histologic) images

Contributed by Roberto N. Miranda, M.D.

Primary C-ALCL

Cytological features

CD4 positivity

Strong CD30 positivity

ALK negativity

Systemic ALCL ALK- in skin

Cytologic features

Anaplastic morphology

Strong CD30+

Positive stains
Negative stains
Molecular / cytogenetics description
Sample pathology report
  • Skin, punch biopsy:
    • CD30 lymphoproliferative disorder, mostly consistent with cutaneous ALK- anaplastic large cell lymphoma, in the appropriate clinical context (see comment)
    • Comment: The section shows an extensive dermal infiltrate composed predominantly of medium to large sized lymphocytes and admixed neutrophils and histiocytes. Scattered mitotic figures are noted. There is epidermal ulceration and extensive neutrophilic serum crust that prevents the evaluation of epidermotropism. Immunohistochemical studies show that the large lymphoid cells are diffusely positive for CD30. CD3 and CD20 label a mixed population of T and B small lymphocytes in the background; the large cells are only focally positive for CD3 and are negative for CD20. The large cells are also partially positive for CD4, while CD8 highlights very rare background lymphocytes. TCR beta labels background lymphocytes while TCR gamma is essentially negative. CD56, EBER and ALK1 are negative.
Differential diagnosis
Board review style question #1

    Which marker is typically negative in primary cutaneous ALCL?

  1. ALK
  2. CD30
  3. CD4
  4. CD45
  5. Perforin
Board review style answer #1
Board review style question #2

    The diagnosis of primary cutaneous ALCL requires that at least what percent of lymphoma cells express CD30?

  1. 20%
  2. 30%
  3. 75%
  4. 90%
  5. 100%
Board review style answer #2
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